188J Maxillofac Oral Surg 8(2):188–191 CASE REPORT

Samprati J Badjate1  · Cariappa KM2 · Ramsay-Hunt syndrome complicating Shenoi SR3 · Shweta Nakhate4 osteonecrosis of edentulous 1 Reader, Dept. of Oral and Maxillofacial Surgery 3 Professor and Vice Dean and : report of a rare case 4 Final BDS Student

VSPM’S Dental College and Research Received: 1 February 2009 / Accepted: 17 May 2009 Center, Nagpur © Association of Oral and Maxillofacial Surgeons of India 2009 2 Professor and Head, Dept. of Oral and Maxillofacial Surgery, Manipal College of Dental Sciences, Manipal Abstract Review of literature revealed atleast 30 cases of post herpes zoster osteonecrosis of maxilla or mandible. To our knowledge this is a first reported case Address for correspondence: of Ramsay-Hunt syndrome with post herpetic neuralgia and post herpes zoster osteonecrosis of edentulous maxilla and mandible. We have briefly reviewed the Samprati J Badjate C/o Dr. J.V. Badjate pathophysiology and management of post herpes zoster osteonecrosis and post Badjate’s Hospital, 39, Ranapratap Nagar herpetic neuralgia. S.E.Rly Colony IInd Layout Nagpur-440022, Maharashtra, India Keywords Herpes zoster · Ramsay-Hunt syndrome · Osteonecrosis · Maxilla · Ph: 09370997210 Mandible · Post herpetic neuralgia E-mail: [email protected] Introduction teeth with of the alveolar mandibular division of trigeminal nerve [13–17]. with ipsilateral ear involvement. Vesicles Herpes zoster (, zona) is an acute A review of literature revealed atleast ruptured to leave raw areas that healed with neurodermic viral infection of the dorsal 30 cases of post herpes zoster osteonecrosis hyperpigmentation initially and root ganglia of the spinal cord or the of either maxilla or mandible. subsequently got depigmented. Intraorally extramedullary cranial nerve ganglia [1] or The simultaneous appearance of the ulcers were present on the left maxillary motor portion of the geniculate ganglion Ramsay Hunt Syndrome has been discussed buccal sulcus and hemipalate. In addition, of the facial nerve. In cases where the facial only twice previously [18,19]. there was extensive of buccal and nerve becomes infected, the clinical We are reporting a case of Ramsay- alveolar mucosa exposing the alveolar bone manifestations are hemifacial paralysis and Hunt syndrome with post herpetic neuralgia in the left mandibular region. He was xerostomia. This condition is termed the and post herpes zoster osteonecrosis of both diagnosed clinically as herpes zoster Ramsay Hunt syndrome [2]. Previous edentulous maxilla and mandible in a 86- infection with facial nerve paralysis and reports have shown that the condition is years-old male patient. treated with antiviral drugs for seven days attributable to reactivation of latent and was referred to department of oral and varicella zoster virus in the dorsal root maxillofacial surgery after 4 weeks for ganglia after an earlier attack of chicken Case report further management. pox [3]. Varicella–zoster virus reactivates Extraoral examination revealed with increasing age or immunosuppression A 86-years-old male patient was referred hypopigmented scar on the left side of the of the infected person; however, the by a private practitioner with complaints face not crossing the midline, extending biologic mechanisms that underlie the of pain during mastication in left maxillary from temporal to symphyseal region (Fig. transition from latency to active viral and mandibular segment, difficulty in 1). Blood crusted areas seen on left tragus. replication are unknown. opening mouth and vertigo since 6 weeks, Low-grade lower motor neuron facial palsy Perhaps most common complication of and decreased hearing from left ear and was present on left side. Intraoral herpes zoster infection is post herpetic lower motor neuron facial palsy on the left examination revealed edentulous upper and neuralgia [4], others include motor nerve side since 3 weeks. Patient was non- lower arches. Oral hygiene was poor with palsy, optic neuropathy, blindness, diabetic, non-smoker and does not abuse marked halitosis. There was extensive encephalitis, and cutaneous calcinosis [5– alcohol. Patient was on antihypertensive fibrosis of the labial and buccal mucosa 8], there are few reports of bony and dental medication since 10 years and negative for with obliteration of the labial and buccal complications by herpes zoster infection, syphilis, HIV and HBsAg. There was no sulcus in the left maxillary and mandibular all of which were isolated in a single history of irradiation, malignancy or trauma region. Depapillation and white scarring of quadrant. These include devitalized teeth in the past. Six week back patient had the left half of the tongue not crossing the [9,10], abnormal development of severe pain and itching on the left side of midline was present (Fig. 2). The alveolar permanent teeth [11], internal resorption the face following which vesicles appeared bone was exposed in the mandibular arch [12], and spontaneous exfoliation of the along the course of left maxillary and from left central incisor to left retromolar

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persists for 40 years after the original attack of the chicken pox [21]. Approximately 8% of patient with HZI have an underlying disease such as leukemia, Hodgkin disease, myelomatosis, or carcinomatosis [20]. Attacks also may be precipitated by surgical stress, immunosuppressive therapy, physical trauma and radiation therapy [13,21,23,24]. In the case presented here there is no known predisposing factor. The trunk (especially T3-L3) and the trigeminal area are most commonly Fig. 1 Showing hypopigmented scar seen in Fig. 2 Showing white scarring of left anterior affected [20,25]. Characteristically herpes relation to left hemiface along the course of half to the tongue zoster of the trigeminal nerve distribution maxillary and mandibular division of manifest as a painful, vesicular eruption trigeminal nerve not crossing midline of the skin and mucosa innervated by the affected nerve. Oral vesicles usually appears after the skin manifestations. The vesicles rupture and coalecse, leaving mucosal erosions. Crusting and healing follow without subsequent scarring in most of the cases. A rare form without vesicular eruption also has been documented [26]. Hunt first described the syndrome of herpes zoster cephalicus, is a rare complication of shingles. The syndrome, caused by the spread of the virus to facial nerves, is characterized by a viral prodrome followed by intense ear pain, a rash around the ear, mouth, face, neck, tongue, larynx, or buccal mucosa and paralysis of facial Fig. 3 Showing completely edentulous Fig. 4 Showing isolated areas of necrosis nerves. The distribution of the vesicles mandibular arch with mucoperiosteal necrosis with exposure of bone in the maxillary left depends on which sensory fibers are and exposure of yellowish necrotic alveolar ridge. Buccal mucosa on the left side infected. Any of the nerve branches that mandibular left alveolar bone extending from is non-elastic communicates with the facial nerve may be midline to retromolar region with no evidence of pus discharge involved, including cranial nerves V, VIII, IX, and X, and cervical nerve II through region and was yellowish in color (Fig. 3). bone was sent for histopathological IV. In the mildest form, neurologic signs In the maxillary arch alveolar bone was examination. The report described areas of are absent, whereas in severe cases there exposed in the left premolar and molar necrotic bone with inflammatory cells with may be accompanying sensorineural region (Fig. 4). There was no evidence of no evidence of fungal or bacterial colonies hearing loss, disturbed vestibular function, pus discharge. ENT consultation revealed (Fig. 6). and even viral encephalitis. sensoneural hearing loss, vertigo and Initial treatment resulted in wound Other symptoms may include loss of secondary infection of left ear. dehiscence and treatment repeated after 3 taste, dry mouth or eyes. The prognosis is Orthopantomogram showed no weeks and same antibiotic course repeated. good. However, in some cases, hearing loss formation or separation of vital Wound healed satisfactorily. may be permanent. Vertigo may last for bone from non-vital bone (Fig. 5). In view days or weeks. Facial paralysis may be of herpes zoster infection of left maxillary temporary or permanent. and mandibular division of trigeminal nerve Discussion The actual pathophysiology of with ear involvement and facial paralysis, osteonecrosis in HZI of the trigeminal nerve a diagnosis of Ramsay Hunt Syndrome Herpes Zoster Infection (HZI) is remains a contentious subject. Various complicated by bimaxillary unilateral predominantly a disease of the middle-aged hypothesis concerning the pathogenesis of alveolar necrosis and post herpetic and elderly. From 5–10 cases per 1000 the herpes zoster-bone necrosis include: neuralgia was made. Hematologically persons are seen between the sixth and eight 1. Local vasculitis caused by direct patient was stable. Patient had undergone decades of life [20], less than 5% of attack extension of the neural inflammatory surgical trimming of exposed bone in upper occurs in persons younger than 10 years of process to the adjacent blood vessels. and lower arch under local anesthesia and age [21,22]. The increasing frequency of This eventually may cause an was started on Amoxicillin 500mg and the HZI with the age has been suggested to of the trigeminal vessels that Metronidazole 400mg thrice-daily be due to the disappearance of zoster- accompany the trigeminal nerves postoperatively for one week. Curetted neutralizing antibodies, which usually supplying the [15,27].

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2. A generalized infection of trigeminal equivalent per day) and an acyclovir nerves supplying the periosteum and medical regimen (200 to 400 mg four periodontium is believed to cause times daily for 7 days) [38]. Medical over a larger area management of chronic PHN consists of [13,14,17]. Garty [16] et al., however topical or systemic therapies [38]. Topical claims that a generalized avascular capsaicin [39] preparations are available. necrosis is not likely, especially in the Systemic therapies of choice are tricyclic maxilla, because of its rich vascular antidepressants [40]. anticonvulsant supply. agents [41], alpha blocking agents [42], 3. Denervation of the bone, which seems and systemic lidocaine [43]. unlikely to cause bone necrosis. Fig. 5 X-ray OPG does not show separation More studies are needed to fully 4. Systemic viral infection can cause of vital bone from non-vital bone understand the pathophysiology of this injury to odontoblasts and cause condition for the purpose of prevention and degenerative tissue changes that result effective management. There is also a need in necrosis [28]. for early detection to prevent the Aseptic osteonecrosis represents a stage complication that has been highlighted. in which portions of the bone are no longer Every case of herpes zoster should be viable. This phenomenon may be a result of closely followed for atleast 6 months to inadequate vascularity of bone. The role of enable early treatment. vascular alteration in the development of bone necrosis is further supported by the fact that osteonecrosis usually occurs in the References patients with compromised vascularity because of ageing, irradiation, or chronic 1. Cooper JC (1977) Tooth exfoliation and . Infection frequently osteonecrosis of the following intervenes and septic osteonecrosis, which herpes zoster. Br Dent J 143(9): 297– represents active infection within bone, 300 occurs. Further, it seems reasonable to 2. Denny-Brown D, Adams RD, assume that preexisting pulpal or periodontal Fig. 6 Photomicrograph showing necrotic Fitzgerald PJ (1994) Pathologic inflammatory conditions have the potential bone with inflammatory cells with no features of herpes zoster. Arch of evidence of fungal or bacterial colonies to contribute to a greater probability of the Neurol Psychiat 51: 216 (10 x 40X hematoxylin and eosin stain) tooth exfoliation and bone necrosis. 3. Barret AP (1990) Herpes zoster virus One of the earliest signs of Accordingly, a mild loss of large fiber fine- infection: A clinicopathologic review associated tactile senses is often measurable on routine and case reports. Aust Dent J 35(4): 328 with a HZI is spontaneous exfoliation of neurosensory examination, especially –332 teeth prior to the overt signs of notable in the loss of vibratory and two- 4. Hess TM, Lutz LJ, Nauss LA, Lamer osteonecrosis. In non-viral osteonecrosis, point tactile sensibilities. The lesions TJ (1990) Treatment of acute herpetic however, dental pain usually heralds the effectively induce a partial deafferentation neuralgia: A case report and review of exfoliation of teeth. This suggests that the in the trigeminal-brain stem complex. There the literature. Minn med 73(4): 37–40 periosteal blood flow is altered in the HZI, is other evidence that either the 5. 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Comprehensive reviews of treatment Ann Dermatol Venereol 122(6–7): Once post herpetic neuralgia disappears, it for acute zoster and PHN have been 436–438 does not recur. PHN does not occur before published [34–37], although no protocol 9. Gregory WB Jr, Brooks LE, Penich EC 50 years of age [31]. PHN is believed to be is universally accepted. The acute (1975) Herpes zoster associated with due to a loss of inhibitory sensory function symptoms are managed aggressively with pulpless teeth. J Endod 1(1): 32–35 with a selective necrosis of larger trigeminal systemic steroids such as Dosepak 10. Goon WW, Jacobsen PL (1988) ganglion cells by zoster viruses [32]. cortisone (5 days course of 20 mg cortisol Prodromal odontalgia and multiple

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